Background: Parahiatal hernia is a less common form of diaphragmatic hernia, with the defect lateral to the crus and oesophageal hiatus, and the unfamiliarity of this disease would cause confusion to general surgeons. In the present study, the literature on parahiatal hernia were reviewed, the clinical profile of this disease, as well as our own experience is presented and analysed. Methods: In the present study, a thorough identification of all published reports on parahiatal hernias was made, together with our own cases, the available data were summarized, analysed and discussed. Results: A total of 27 cases of parahiatal hernias were identified since 1987. Among them, 19 cases were primary parahiatal hernias, and eight cases were secondary or acquired parahiatal hernias. None of the 27 cases were pre-operatively diagnosed, and the majority of them were pre-operatively diagnosed as paraoesophageal hernias. Detailed treatment data were available in 26 of the 27 cases. Three patients received open surgery, and 23 patients were treated with laparoscopic procedures. Suture repair was used in 12 cases, and 14 cases were repaired with mesh reinforcement. In addition, two cases underwent partial gastrectomy, stomach suture was performed in another two cases. Conclusion: Patients with parahiatal hernia have a high risk of developing hernia incarceration or gastric vovulus. Laparoscopic treatment of parahiatal hernia is feasible and safe in the majority cases. Surgeons should be aware of this disease when performing paraoesophageal hernia repair, as parahiatal hernias may occur with or without previous diaphragmatic surgery.